MATURITY ONSET DIABETES OF THE YOUNG (MODY)

This is rare form of type 2 diabetes inherited in an autosomal dominant fashion and presenting at young age. It comprises <5% of childhood diabetes and <1% of all type of diabetes. It is also known as Mason-type diabetes after the index family. The diagnostic criteria include diagnosis of type 2 diabetes with onset before age of 25 years in at least two family members, with evidence of vertical transmission throughout at least three generations.

All 6 types of MODY are characterized by defects in insulin secretion rather than insulin action, but sometimes insulin resistance is seen due to glucotoxicity. The defect consists of mutation in gene in β cells, encoding glucokinase (MODY 2) and other transcription factors leading to (MODY 1-6) which leads to defective insulin action, pancreatic dysfunction, etc.